Treatment of systemic scleroderma and generalized morphoea with coagulation factor XIII

A. V. Marzano, G. Gasparini, C. Colonna, E. Berti, R. Caputo

Research output: Contribution to journalArticlepeer-review

Abstract

In the blood coagulation process, factor XIII is responsible for the stabilization of the fibrin clot. Thivolet et a. [10] have used this factor in the treatment of scleroderma, hypothesizing as to its ability to favour the degradation of the collagen that accumulates in the skin and in other affected organs. Based on these findings and on the in vitro studies of Paye et al. [24], we administered factor XIII to twelve patients, eight of whom were suffering from sys temic scleroderma, three from generalized morphoea and one from localized morphoea. All had severe, widespread skin sclerosis. The mean duration of treatment was 35.5 months. Both short- and long-term drug safety was excellent. After approximately ten months of treatment, skin sclerosis, as evaluated by a semiquantitative method, was markedly improved in 75% of the cases. Stabilization of the clinical signs and symptoms was also observed and in two cases of generalized morphoea continued administration was regarded as unnecessary. On the other hand, the efficacy of factor XIII on organ involvement proved unclear even though its administration in combination with traditional drugs, such as low-dose corticosteroids or D-penicillamine, seemed to improve respiratory function in several cases As a consequence, factor XIII can be propsed both as the choice treatment in generalized morphea accompanied by ingravescent skin sclerosis, and as a drug to be combined with traditional treatments in systemic scleroderma.

Original languageEnglish
Pages (from-to)459-466
Number of pages8
JournalEuropean Journal of Dermatology
Volume5
Issue number6
Publication statusPublished - 1995

Keywords

  • Factor XIII
  • Generalized morphoea
  • Localized morphoea-systemic scleroderma

ASJC Scopus subject areas

  • Dermatology

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